Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges

Curr Probl Cancer. 2021 Aug;45(4):100768. doi: 10.1016/j.currproblcancer.2021.100768. Epub 2021 Jul 1.


Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRβ, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.

Keywords: Inflammatory myofibroblastic tumor; molecular profiling; pediatrics; rare tumor; targeted therapy, crizotinib, ALK inhibition.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Antineoplastic Agents / adverse effects
  • Antineoplastic Agents / therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Molecular Targeted Therapy
  • Neoplasms, Muscle Tissue* / diagnosis
  • Neoplasms, Muscle Tissue* / drug therapy
  • Neoplasms, Muscle Tissue* / genetics
  • Neoplasms, Muscle Tissue* / surgery
  • Pyrimidines / adverse effects
  • Pyrimidines / therapeutic use
  • Sulfones / adverse effects
  • Sulfones / therapeutic use
  • Treatment Outcome


  • Antineoplastic Agents
  • Pyrimidines
  • Sulfones
  • ceritinib